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A 66-year-old man presented with dull, left-sided chest pain for 1 week occurring mainly at rest. The symptoms had been present for 1 month but had increased in frequency over the preceding 3 days. He became acutely short of breath with substernal chest pain while climbing a flight of stairs the day of admission. The pain was pressure-like, did not radiate and was associated with presyncope, but not syncope, diaphoresis, nausea nor vomiting. He had no past medical history of cardiac disease but did have risk factors for coronary artery disease (CAD) including hypertension, history of smoking and the metabolic syndrome. His past medical history was also significant for non-Hodgkin's stage IV marginal zone B cell lymphoma treated with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) 4 years earlier. Vital signs and physical exam were normal without murmurs, rubs or gallops. The lung fields were clear, there was no jugular venous distention nor lower extremity oedema. A 12-lead ECG showed normal sinus rhythm without ST segment elevations and his serum troponin I was elevated to 1.69 ng/mL. He was treated medically for a non-ST segment elevation myocardial infarction and underwent non-emergent coronary angiography for recurrent chest pain. Figure 1 shows injection of right coronary artery (RCA) in left anterior oblique orientation (LAO) at the time of coronary angiography (figure 1).
Cardiac angiographic image is most consistent with the following:
Pulmonary arterial-venous malformation with fistula to the RCA.
Significant stenosis of the mid portion of the RCA.
Tumour blush due to vascular tumour with blood supply derived from the RCA.
Fistula between the RCA and right atrium (RA).
From the question on page 1619
Angiogram of the RCA showed tumour blush in the RA (figure 2A), not the pulmonary artery nor lung, as expected in a pulmonary artery fistula. RCA-RA fistula would opacify the entire atrium instead of a discrete area. There is no RCA stenosis and the left coronary artery had non-obstructive disease. Cardiac MRI and transesophageal echo confirmed a soft tissue mass within the RA (figure 2B,C). The mass was surgically resected (figure 2D) and showed high-grade angiosarcoma (figure 2E). The patient underwent chemotherapy, surviving 8 months.
Contributors All authors had a highly significant contribution to this work. RDL drafted the article and collected images. MP, JDV and DRM collected additional surgical and pathological images and contributed significantly to manuscript revisions. GK conceptualised the format, contributed significantly to revisions and obtained clinical images.
Competing interests None.
Provenance and peer review Not commissioned; internally peer reviewed.